tbuckley/cabot-search huggingface dataset:
POS0774 CESAREAN SECTION IN WOMEN WITH JUVENILE IDIOPATHIC ARTHRITIS – A POPULATION-BASED STUDY
Authors: C. Skorpen, S. Lydersen, K. Å. Salvesen, M. Wallenius
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> The literature on delivery methods in women with juvenile idiopathic arthritis (JIA) is insufficient. Active inflammation is a risk factor for cesarean section (CS) in other arthritic diseases. A CS entails a higher risk for complications than vaginal delivery and a restricted physical activity the first weeks after birth. <h3>Objectives</h3> To explore a possible association of inflammatory active disease and the proportion of CS in women with JIA. <h3>Methods</h3> Data from the Norwegian nationwide observational register RevNatus were linked with data from the Medical Birth Registry of Norway (MBRN). Cases comprised singleton births in women with JIA (n= 196) included in RevNatus 2010 to 2019. Singleton births registered in MBRN during the same period of time and excluding mothers with rheumatic inflammatory diseases (n = 575 798), served as population controls. Disease activity was assessed using Disease Activity Score with CRP (DAS28-CRP-3). We defined inactive JIA as DAS28-CRP-3 &lt; 2.6 and active JIA as DAS28-CRP-3 ≥ 2.6. <h3>Results</h3> CS was more frequent in women with JIA (20.4%) than in population controls (15.6%) and occurred most frequently in inflammatory active JIA (30.0%). Women with JIA had similar risk for elective CS (risk difference 1.1%, 95% CI-1.7 to 5.4) and higher risk for emergency CS (risk difference 3.8%, 95% CI -0.4 to 9.3) compared with population controls. Active disease increased the risk for emergency CS (risk difference 14.0%, 95% CI 4.3 to 27.4). <h3>Conclusion</h3> Women with active JIA had higher risk for emergency CS compared with population controls. <h3>Reference</h3> [1]Remaeus K, Johansson K, Askling J, et al. Juvenile onset arthritis and pregnancy outcome: a population-based cohort study. <i>Ann Rheum Dis</i> 2017;76(11):1809-14. doi: 10.1136/annrheumdis-2016-210879 [published Online First: 2017/07/01] <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS0717 COMPARISON BETWEEN ORAL AND INTRAMUSCULAR GLUCOCORTICOID THERAPEUTIC SCHEME IN POLYMYALGIA RHEUMATICA: INTERIM ANALYSIS OF PROSPECTIVE SINGLE-CENTRE STUDY
Authors: J. Kramarič, S. Markez, A. Hočevar, Z. Rotar, A. Ambrožič
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Many patients with polymyalgia rheumatica (PMR) require prolonged glucocorticoid (GC) treatment, resulting in high cumulative GC doses. Treatment with intramuscular (IM) methylprednisolone (MP) injections results in considerably lower cumulative dose than conventional oral GC. <h3>Objectives</h3> We aimed to evaluate clinical and laboratory response and relapse frequency between PMR patients treated with oral or IM GC regimens. <h3>Methods</h3> This prospective study included patients with newly diagnosed PMR between October 2021 and September 2022. At diagnosis, patients were randomly allocated to IM or oral MP dosing groups in 1:2 ratio. Both groups followed a predefined MP tapering regimen. IM injections of 120 mg of MP were given every 3 weeks for the first 16 weeks, then every 4 weeks with dose reduction by 20 mg every 12 weeks until 60 mg, then by 20 mg every 16 weeks. Oral MP was started at 16 mg/day then, every 4 weeks, the dose was reduced to: 12 mg, 8 mg, 6 mg and 4 mg. Follow-up visits were performed adhering to protocol. The remission rate and other measures of disease activity were recorded and compared between IM MP and oral MP groups. Descriptive statistics were used to analyse the studied population. <h3>Results</h3> Of the 39 PMR patients (72% female, median (IQR) age 71 (66, 76) years) 26 patients received oral MP and 13 IM MP. Results are presented in Table 1. During follow-up we observed higher ESR values in the IM MP group. We observed slower clinical response in IM MP group at 4 weeks of follow-up. There was no difference between the two groups in clinical response measures at 12 and 24 weeks. During 24 weeks of follow-up, we identified 2 relapses in the oral MP group: 1 patient relapsed when lowering the MP dose to 6 mg/day at 16 weeks and 1 relapsed after discontinuing MP at week 24 due to patient’s choice and presented with symptoms of giant cell arteritis. In the IM MP group, we observed no relapses in the first 24 weeks of follow-up, but there were 4 patients with inadequate response to treatment: 2 at week 4 and 2 at week 12. In these cases, we switched to oral MP. Due to the low number of patients in our study, results should be interpreted with caution. <h3>Conclusion</h3> In our cohort, treatment with IM MP compared to oral MP was effective in the majority of patients with PMR, but was associated with higher inadequate response rate and overall slower clinical and laboratory response. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS0727 DEVELOPMENT OF POSTERIOR UVEITIS IN BEHÇET’S SYNDROME PATIENTS WITH VITREOUS CELLS WITHOUT ANY OTHER POSTERIOR INVOLVEMENT
Authors: D. Ucar, B. E. Bircan, N. Rustamli, B. Batu Oto, V. Hamuryudan, S. N. Esatoglu, G. Hatemi
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> A considerable number of patients with Behçet's syndrome (BS) have vitreous cells on slit lamp examination at the time of diagnosis. However, the prognostic importance of vitreous cells (VC) and their association with the development of posterior uveitis (PU) requiring immunosuppressive treatment is unknown. <h3>Objectives</h3> We aimed to determine the prognostic importance of VC in BS patients. <h3>Methods</h3> The charts of 572 consecutive BS patients fulfilling ISG criteria who were registered between 2010 and 2012 were reviewed. At baseline visit 164 patients had VC in one eye or both eyes. Among the remaining patients, 229 had no eye involvement, 116 patients had bilateral pan or posterior uveitis, 14 had unilateral pan or posterior uveitis and no eye involvement in the other eye, 20 had isolated anterior uveitis, and 29 had insufficient data in their medical records. Among the 164 patients with VC, 110 patients with a follow-up of ≥2 years were included in this study (Figure 1). <h3>Results</h3> At baseline, among the 111 included patients (68 men, mean ± SD age: 31±9 years), 61 had VC in both eyes, 34 had VC in only one eye, and 15 had VC in one eye and PU in the other eye. There was anterior uveitis (AU) in addition to VC in the same eye in 13 patients at baseline (Figure 1). New PU developed in 26 (24%) patients during a mean follow-up of 1.9±1.1 years.7 patients that developed PU in the eye with VC had had PU in the contralateral eye at baseline. This means 7 of 15 patients with VC in one eye and PU in the contralateral eye developed bilateral PU despite treatment. 2 of these 7 patients had developed AU before they developed PU. Additionally, 6 patients that developed PU in the eye with VC had anterior uveitis in the same eye at baseline. <h3>Conclusion</h3> Careful follow-up is required for patients with VC since one quarter developed PU within 2 years. The presence of PU in the contralateral eye and AU in the same eye may be risk factors for the development of PU in patients with VC. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS0746 CONTRIBUTION OF GENETIC VARIANTS ASSOCIATED WITH PRIMARY IMMUNODEFICIENCIES IN CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOUS PATIENTS
Authors: C. Y. Wu, W. L. Fan, P. S. Chu, P. C. Liao, W. I. Lee, J. L. Huang
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Dysregulated immune response is the hallmark of autoimmune disorders[1]. Evidences suggested that systemic autoimmune diseases and primary immunodeficiency disorders (PID) may be similar diseases with alternative clinical phenotypes[2]. <h3>Objectives</h3> This study aimed to investigate the burden of PID associated genetic variants in the childhood-onset systemic lupus erythematous (cSLE) patients. <h3>Methods</h3> One hundred and eighteen cSLE cases regularly followed at Chang-Gung memorial hospital were enrolled. Targeted next-generation sequencing was applied searching for PID genetic variants. Genetic variants were compared to those of 1,475 unrelated healthy individuals and further filtered by allelic frequency and various functional score systems. Customized immune assays were applied to test the function of the identified variants. <h3>Results</h3> Upon filtration, 36 (30.5%) patients harbored rare variants in PID-associated genes predicted to be damaging. One homozygous <i>TREX1</i> (c.294dupA) mutation and four heterozygous variants with possible dominant PID traits, including <i>BCL11B</i> (c.G1040T), <i>NFKB1</i> (c.T695G), and <i>NFKB2</i> (c.G1210A, c.G1651A), were discovered. With recessive traits, variants were found across all PID types; one-fifth involved phagocyte number or function defects. Predicted pathogenic PID variants were more predominant in those with a family history of lupus regardless of infection susceptibility. Moreover, mutational loads were greater among cSLE patients than controls despite sex or age at disease onset. While greater mutational loads were observed among cSLE patients with peri-pubertal disease onset, no significant difference in sex or phenotype were noted among cSLE patients. <h3>Conclusion</h3> Our findings suggest that cSLE is mostly not a monogenic condition. Gene-specific analysis and mutational load investigations suggested that while rare and predicted damaging variants in immunodeficiency related genes may contribute to cSLE susceptibility, genetic investigations of PID disorders may not be warranted for cSLE patients with recurrent infections. <h3>References</h3> [1]Kaul A, Gordon C, Crow MK, Touma Z, Urowitz MB, van Vollenhoven R, et al. Systemic lupus erythematosus. Nat Rev Dis Primers. 2016;2:16039. [2]Grammatikos AP, Tsokos GC. Immunodeficiency and autoimmunity: lessons from systemic lupus erythematosus. Trends Mol Med. 2012;18(2):101-8. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS0669 CROSS-SECTIONAL ANALYSIS OF CARDIOVASCULAR DISEASE AND RISK FACTORS IN PATIENTS WITH SPONDYLOARTHRITIS: A REAL-LIFE EVIDENCE FROM BIOSTAR NATIONWIDE REGISTRY
Authors: M. T. Duruöz, H. Bodur, Ş. Ataman, G. Gürer, Ö. Akgül, H. F. Cay, E. Capkin, İ. Sezer, A. Rezvani, M. Alkan Melikoglu, İ. Yağci, F. G. Yurdakul, F. N. Göğüş, A. Kamanli, R. Çevik, L. Altan İnceoğlu
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Association between spondyloarthritis and cardiovascular (CV) diseases is a complex issue with variable outcomes. <h3>Objectives</h3> This study aimed to assess the prevalence rates of CV diseases and to analyze the impact of CV risk factors on CV disease in patients with spondyloarthritis. <h3>Methods</h3> A multi-center cross-sectional study using BioSTAR (Biological and Targeted Synthetic Disease-Modifying Antirheumatic Drugs Registry) database was performed on patients with spondyloarthritis. Socio-demographic, laboratory and clinical, data were collected. Patients with and without major adverse cardiovascular events (MACE) were grouped as Group 1 and Group 2.The primary outcome was the prevalence rates of CV disease and CV risk factors in the overall group. The secondary outcome was the difference in socio-demographic and clinical characteristics between the groups and predictive risk factors for CV disease. <h3>Results</h3> There were 1457 patients with a mean age of 45.7±10.9 years. The prevalence rate for CV disease was 3% (n=44). The distribution of these diseases was coronary artery disease (n=42), congestive heart failure (n=4), peripheral vascular disorders (n=6), and cerebrovascular events (n=4). Patients in Group 1 were significantly older than those in Group 2 (p&lt;0.001). There were significantly more patients with hypertension, diabetes mellitus, chronic renal failure, dyslipidemia, and malignancy in Group 1 than in Group 2 (p&lt;0.05). Smoking (36.7%), obesity (24.4%), and hypertension (13.8%) were the most prevalent traditional CV risk factors. Hypertension (HR=4.994, 95% CI:1.966-12.683, p=0.001) and dyslipidemia (HR=1.960, 95% CI:1.155-6.676, p=0.024) were the independent predictors for CV disease. <h3>Conclusion</h3> The prevalence rate of CV disease was 3.0% in patients with spondyloarthritis. Hypertension and dyslipidemia were independent CV risk factors for CV disease in patients with spondyloarthritis. <h3>References</h3> [1]Chen CH, Chen HA, Liu CH, Liao HT, Chou CT, Chen CH. Association of obesity with inflammation, disease severity and cardiovascular risk factors among patients with ankylosing spondylitis. Int J Rheum Dis. 2020 Aug;23(9):1165-1174. [2]Kim JH, Choi IA. Cardiovascular morbidity and mortality in patients with spondyloarthritis: A meta-analysis. Int J Rheum Dis. 2021 Apr;24(4):477-486. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS0522 UTILIZATION OF A GOUT ANALYZER (U-GAN) FOR CRYSTAL EXAMINATION IN CLINICAL PRACTICE
Authors: T. Kise, N. Yokogawa, Y. Miyoshi, M. Utsunomiya, Y. Nagai, K. Shimada
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Synovial fluid (SF) crystal analysis performed with a compensated polarized microscopy is the gold standard for identifying calcium pyrophosphate (CPP) and monosodium urate (MSU) crystals. Since a fully equipped compensated polarized microscope is not widely available, a gout analyzer (U-GAN, Olympus) is alternatively used in many countries. By attaching the analyzer in a slider-compatible revolving nosepiece, examination using compensated polarization can be easily performed by operating the lever. <h3>Objectives</h3> To evaluate the utility of crystal examination using U-GAN®. <h3>Methods</h3> We prospectively performed different crystal identification methods in 30 patients with knee joint effusion at Tokyo Metropolitan Tama Medical Center from February 2020 to March 2021. Rheumatologists who routinely use compensated polarized microscope blindly evaluated the non-centrifuged SF using three methods: 1) morphologic identification under ordinary light, 2) use of U-GAN®, and 3) use of fully-equipped compensated microscope (MT9300, Meiji Techno) in this order. In addition, in-hospital commercial laboratory technicians evaluated the sediment using U-GAN® after centrifugation (3000 rpm for 5 min). <h3>Results</h3> Total 30 SF were evaluated. By compensated polarized microscope, 15 were found crystal-positive (11 for CPP and 4 for MSU). The detection of both CPP and MSU by the in-hospital laboratory technician were in perfect agreement. Before centrifugation, the sensitivity of ordinal microscope and U-GAN® were 73.3% (11/15) and 80.0% (12/15), respectively (specificity 100% in both). When using U-GAN®, 3 (2 CPP and 1 MSU) were additionally detected after centrifugation. <h3>Conclusion</h3> U-GAN® can be used for detection of both CPP and MSU in clinical practice. <h3>References</h3> [1]Lumbreras B et al. <i>Ann Rheum Dis</i> 2005;64(4):612-5. [2]Pascual E et al. <i>Curr Opin Rheumatol.</i> 2011;23(2):161- 9. [3]Boumans D et al. <i>Clin Rheumatol</i>. 2017;36(7):1599-1605. [4]Meyer M et al. <i>Int J Rheum Dis</i> 2021;24(1):10-20. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> Takayasu Kise Grant/research support from: Daiichi Sankyo Co. Ltd., Naoto yokogawa Grant/research support from: Daiichi Sankyo Co. Ltd., Yuji Miyoshi Grant/research support from: Daiichi Sankyo Co. Ltd., Masako Utsunomiya Grant/research support from: Daiichi Sankyo Co. Ltd., Yoshiki Nagai Grant/research support from: Daiichi Sankyo Co. Ltd., Kota Shimada Grant/research support from: Daiichi Sankyo Co. Ltd.
POS0703 RENAL ARTERY STENOSIS IS NOT ASSOCIATED WITH WORSE SURVIVAL IN TAKAYASU ARTERITIS – DATA FROM A SINGLE-CENTER RETROSPECTIVE COHORT OF 195 PATIENTS
Authors: D. P. Misra, P. Mishra, K. Singh, U. Rathore, D. Thakare, N. Jain, M. Ora, M. Behera, S. Gamhbir, D. Bhadauria, S. Kumar, V. Agarwal
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Renal artery stenosis (RAS) is more frequent in Asian patients with Takayasu arteritis (TAK). Few studies have assessed the impact of RAS on the presentation and prognosis of TAK using adjusted analysis. <h3>Objectives</h3> To compare the presentation and prognosis between TAK with or without RAS. <h3>Methods</h3> We compared the clinical presentation and vascular involvement (including angiographic subtypes by Hata's classification) between patients with TAK with or without RAS, with bilateral versus unilateral RAS, and with bilateral RAS versus those without RAS using multivariable-adjusted odds ratios (aOR) with 95% confidence intervals (95%CI) derived after logistic regression. We compared survival between these groups using hazard ratios (HR) with 95%CI adjusted for gender, age at disease onset, delay to diagnosis, baseline disease activity, and inter-group differences. The institute ethics committee provided a waiver of consent for retrospective data retrieval. The institute ethics committee provided a waiver of consent for retrospective data retrieval. <h3>Results</h3> Out of 195 TAK with imaging data available, 106 had RAS [58 bilateral, 48 unilateral; mean(±SD) follow-up 41.50(±43.35) months]. TAK with RAS (mean age 22.99 years) or bilateral RAS (mean age 22.83 years) were younger than without RAS (mean age 28.00 years). TAK with RAS had more hypertension (aOR 4.46, 95%CI 1.81 – 10.99), lower limb claudication (aOR 2.72, 95%CI 1.08 – 6.88), and less frequent upper limb claudication (aOR 0.43, 95%CI 0.19-0.99) and syncope or dizziness (aOR 0.35, 95%CI 0.14 – 0.85) than TAK without RAS. TAK with bilateral RAS had more frequent hypertension (aOR 11.83, 95%CI 1.38-101.16), blurring of vision (aOR 5.97, 95%CI 1.02 – 34.83), and less frequent constitutional symptoms (aOR 0.18, 95%CI 0.06 – 0.51) than those with unilateral RAS. TAK with bilateral RAS had more frequent hypertension (aOR 8.73, 95%CI 1.90-40.06), vascular bruits (aOR 2.94, 95%CI 1.03 – 8.34), and heart failure (aOR 4.16, 95%CI 1.00 – 17.32), and less frequent constitutional symptoms (aOR 0.23, 95%CI 0.09 – 0.62), pulse loss (aOR 0.29, 95%CI 0.11 – 0.77), and syncope or dizziness (aOR 0.21, 95%CI 0.06-0.73) than those without RAS. Hata's type IV and type V were more frequent in TAK with versus without RAS (OR 4.01 and 13.40, respectively), and in those with bilateral RAS versus no RAS (OR 5.97 and 10.94, respectively). Adjusted survival was similar between TAK with or without RAS, with unilateral or bilateral RAS, or with bilateral RAS vs those without RAS (Table 1). <h3>Conclusion</h3> RAS is associated with specific clinical and angiographic features but not with a greater risk of mortality in TAK. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS0533 RISK OF SEVERE INFECTIONS IN IMMUNE MEDIATED INFLAMMATORY DISEASES WITH IMMUNOGLOBULIN DEFICIENCY UNDER RITUXIMAB THERAPY
Authors: J. Morel, C. Rempenault, T. Lea, C. Daien, B. Combe, P. Guilpain, C. Lukas
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Rituximab (RTX) is effective in treating immune-mediated inflammatory diseases (IMID). Hypogammaglobulinemia may occur under RTX and may increase infection risk. <h3>Objectives</h3> Since data are controversial, we evaluated the risk of severe infection in patients with IMID and hypogammaglobulinemia under RTX therapy. <h3>Methods</h3> We conducted a retrospective single-center study retrieving all patients treated with RTX for IMID. We calculated the incidence of RTX-induced immunoglobulin (Ig) deficiency, the type of Ig deficiency, the incidence of severe infection, and risk factors for severe infection. <h3>Results</h3> 311 patients were analyzed. Mean follow-up was 62.6 months. Exposure was 1623.7 patient-years. 15% of patients developed at least 1 severe infection. Incidence rate was 2.77/100 PY. 29 patients had prevalent Ig deficiency before being treated with RTX. 68 patients developed hypogammaglobulinemia, mainly for IgM (12%) and IgG (8%). Severe infection rate was higher in patients with prevalent Ig deficiency (RR 1.73; 95% CI 0.85-3.53), with significant difference in survival model (Log-Rank test: p=0.033). On the other hand, no excess risk of infection was observed in patients developing Ig deficiency under RTX in univariate analysis (RR 0.68; 95% CI 0.31-1.47) or in survival analyses after adjustment for confounding factors (type of IMID (RA versus other IMID), cumulative dose of RTX, presence of chronic pulmonary comorbidities, use of an immunosuppressive drug at inclusion, and mean dose of GCs collected at each cycle of RTX during follow-up) (Log-Rank test: p=0.399). Chronic lung disease and glucocorticoids (GCs) use during follow-up were associated with an increased risk of severe infection. <h3>Conclusion</h3> We did not observe an increased risk of severe infection in RTX-induced Ig deficiency. However, we found an increased risk of severe infection in case of prevalent Ig deficiency prior to RTX. In case of Ig deficiency, RTX management should be discussed on a case-by-case basis, according to an individual assessment of the infectious risk, especially when GCs therapy is used and chronic lung diseases are present. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.